2012 Hydrocephalus Association Conference Complex ... · Hydrocephalus! Marion L. Walker, MD!...

Complex Hydrocephalus

Marion L. Walker, MD Professor of Neurosurgery & Pediatrics

Primary Children’s Medical Center University of Utah Salt Lake City, UT

2012 Hydrocephalus Association Conference Washington, DC - June 27-July1, 2012

Complex Hydrocephalus�

Difficult Problems Often Requiring Multiple Shunts�

!   Slit Ventricle Syndrome�

!   Dandy-Walker Syndrome�

!   Multiloculated Hydrocephalus�

!   Septal Fenestration �

!   Avoiding an additional shunt �


Slit Ventricle Syndrome�

!   Headaches�

!   Varying degrees of lethargy�

!   +/- nausea/vomiting �

Intermittent symptoms of shunt malfunction in a child who appears otherwise healthy�


Pathophysiology of� Slit Ventricle Syndrome�

!   Shunt over drainage of CSF�

!   Growing brain fills the intracranial space�

!   a fixed skull filled with brain parenchyma, blood, meninges, vasculature and only small amounts of CSF�

!   Loss of normal intracranial compensatory mechanisms�


!  SVS is a phenomenon occurring in children.�

!  Adults do not get SVS.�

Pathophysiology of� Slit Ventricle Syndrome�



Treatment of the�Slit Ventricle Syndrome�

!   Observation �

!   Limit over drainage�

!   Antimigrainous therapy�

!   ICP monitoring & EVD placement �

•  Shunt revision

•  Subtemporal decompression

•  Cranial morcellation

•  Third ventriculostomy


Slit Ventricle Syndrome�Signs & Symptoms�

!   Overdrainage�

!   Positional headaches�

!   Relief by lying down �

!   Poor tolerance of school�

!   May be asymptomatic for prolonged periods�

!   Slit ventricles are relatively common in patients shunted in childhood.�

!   SVS is not as common and occurs only in patients shunted in childhood.�

!   Treatment options include observation, medication, shunt revision and third ventriculostomy.�

!   No specific treatment or shunt type has been shown to be the best option.�

Slit Ventricle Syndrome Conclusions

Dandy Walker Syndrome�

!   Dandy-Walker Syndrome (DWS) is determined by three findings: �

1.  cystic dilatation of the fourth ventricle�

2. total or partial aplasia of the cerebellar vermis�

3. supratentorial hydrocephalus�


Dandy Walker Syndrome�

!   Dandy-Walker Variant (DWV): �1. the floor & lateral walls of

the 4th ventricle are visible�2. the vermis is hypoplastic�



Differential Diagnosis�!  DWS must be differentiated from an arachnoid cyst of the posterior fossa�


Pathogenesis�!   DWS develops at about the fourth

week of gestation �

!   Associated with other anomalies: �

!   partial or total agenesis of the corpus callosum�

!   many genetic disorders�

!   facial�

!   cardiac�


Incidence of�Dandy Walker Syndrome�

!   1:25,000 - 35,000 births�

!   Female:Male 1.3:1 �

!   80% diagnosed before 1 year of age�

!   Dandy Walker Variant may be an incidental finding �


Incidence of Hydrocephalus�

!   DWS occurs in 2-4% of cases of hydrocephalus�

!   90% of cases of DWS have enlarged ventricles and/or hydrocephalus�


Associated Anomalies�

!   CNS anomalies occur approximately 70%�

!   agenesis of the corpus callosum (40%)�

!   encephaloceles (17%)�

!   heterotopias�

!   aqueductal stenosis�


Associated Anomalies�!   Non-CNS anomalies include: �

!   Cardiac�!   ventricular septal defects�!   patent ductus arteriosus�!   arterial septal defects�

!   External defects often associated with genetic disorders�

!   trisomies 18, 21, and 13�!   <2% recurrence risk �


Signs & Symptoms�

!   Macrosomia (C-section rate 26%)�

!   Delayed motor development �

!   Cognitive dysfunction 40-70%�

!   Focal motor findings very low �

!   Almost no cerebellar dysfunction �

Imaging Studies�CT Scan � MRI �



Surgical Treatment �

!   Cyst removal�

!   failure rate >75%�

!   Shunting for hydrocephalus�

!   cyst?�

!   ventricle?�

!   both? �

Multiloculated Hydrocephalus�Complex Hydrocephalus�

!   Some form of guidance is necessary�

!   ultrasound �

!   stereotaxic frame�

!   frameless�

Multiloculated Hydrocephalus�



!   Often seen in patients with post-hemorrhagic or post-infectious hydrocephalus.�

!   Often have required multiple shunts unless endoscopic techniques are successful.�


• The most difficult cases! �

• Many technical difficulties�

• The poorest outcomes�

• 45% successful shunt avoidance�


Frameless Stereotaxy�

Maintain Intracranial Orientation �


!   Use guidance techniques liberally�

!   Ultrasound�

!   Stereotaxis�

Maintain Intracranial Orientation �

Frameless Stereotaxy�


Isolated Lateral Ventricular Hydrocephalus �

• Etiology: �• Neoplastic: e.g. hypothalamic glioma�

• Congenital: e.g. atresia of Foramen of Monroe�

• Benign lesions: choroid plexus cyst or hypertrophy �

• Post infectious or hemorrhagic scarring �

• Iatrogenic: unilateral shunt overdrainage�

!   Clinical signs of increased ICP�

!   Radiographic: Ventricular asymmetry + �

!   Non-communication of intraventricular contrast �

!   Progressive monoventricular enlargement �

!   Known mass obstructing Foramen of Monroe�

Isolated Lateral Ventricular Hydrocephalus �



Treatment Outcome�!   Success: �

!   On last follow-up: Absence of ILVH �

!   Failure: �

!   Recurrence of ILVH symptoms & radiographic evidence�

Total Successes: 26� Total Failures: 6�n=32�

Patients re-operated�1 �

Patients not re-operated�1 �

3rd septostomy�

Success�1 �

Patients not re-operated�5�

Patients re-operated�10�

2nd septostomy�

Success�8�

Failure�2�

First septostomy�

Success�17�

Failure�15�


Success Rates�!   All patients: " 81.2%

(27/32)�

!   All septostomies: 60.5% (26/43)�

!   First septostomies: 53.1% (17/32)�

!   Redo patients: " 90% (9/10)�

Septostomy: Survival Curve�

Prop

ortion

fai

lure

-fre

e�

Months follow-up �

*No failures occurred after 6 months�

* �

Drake, et al. Neurosurgery 43: 294-305, 1998�

Septostomy vs. VP Shunt �


•  Endoscopic septostomy provided long term ILVH relief in 81% of patients

•  After 6 months post-op, septostomy failures not observed

•  2 or more prior shunt procedures negatively affects outcome

•  Good results possible in redo septostomies

•  A reasonable alternative to CSF shunting in cases of isolated lateral ventricle hydrocephalus

Conclusions


Avoid That Second Shunt! �

!   Communicate intraventricular cysts�

!   Communicate through the septum pellucidum for isolated ventricles or unilateral hydrocephalus�


Complex Hydrocephalus Conclusions�

!   Management decisions for difficult shunt problems are best approached in a systematic manner.�

!   Successful treatment of the slit ventricle syndrome is often accomplished by conservative management.�

!   Several surgical attempts may be necessary in order to avoid additional shunts in patients with loculated ventricles.�

2012 Hydrocephalus Association Conference Complex ... · Hydrocephalus! Marion L. Walker, MD!...

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